HAE Patient or Family Caregiver Resources

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Learning you or a loved one has been diagnosed with hereditary angioedema (HAE) can be very unsettling. It’s a rare condition most people have never heard of. That’s why it’s important to know both what’s happening in the body during an HAE attack and your options for managing the disease.

HAE is a disease that has as its root cause a defect in C1-inhibitor activity, usually as a result of a genetic condition. It affects about 1 in 10,000 to 1 in 50,000 people. Lack of C1-inhibitor leads to uncontrolled activity of an enzyme known as plasma kallikrein, which triggers excessive release of a peptide called bradykinin. This in turn can lead to episodic attacks of swelling (edema) in the hands, feet, gastrointestinal tract, face and larynx (airway).

An HAE episode with abdominal or intestinal wall swelling can cause severe pain, nausea and vomiting. Swelling that attacks the larynx can lead to suffocation and potentially life-threatening situations.

What to expect

Inflammation in the body is often linked to stress or physical activity, but not always. C1- inhibitor deficiency can also contribute to inflammation. That helps explain why some HAE attacks seem to occur spontaneously, with no apparent trigger.

HAE attacks are typically prevented or treated by replacing the missing C1-inhibitor protein or by other therapies that block the activity of plasma kallikrein or bradykinin. All the currently approved HAE therapies are delivered by injection.

How and where to get treatment for HAE

Attacks can typically be prevented or treated by replacing the missing protein through regular injections or by using other therapies that block the pathway that leads to the sudden swelling attacks. Because all the current therapies are injected or infused (given intravenously), they are less convenient to use and sometimes require additional assistance. KalVista is developing an oral medication that would be easier for patients to take in the early stages of an attack, which we believe could shorten the duration and severity of the swelling.